NR APHT
AU Haik,S.; Faucheux,B.A.; Sazdovitch,V.; Privat,N.; Kemeny,J.L.; Perret-Liaudet,A.; Hauw,J.J.
TI The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease
QU Nature Medicine 2003 Sep; 9(9): 1121-3
PT case reports; journal article
AB Prion epizoonoses spread from animals consumed by humans raise the question of which pathways lead to prion neuroinvasion after oral exposure of humans. Here we show that neurons of sympathetic ganglia of patients with variant Creutzfeldt-Jakob disease (vCJD) accumulate the abnormal isoform of the protein prion. This observation shows the involvement of the sympathetic nervous system in the pathogenesis of vCJD and suggests a role for GUT-associated sympathetic neurons in prion propagation in humans after oral contamination.
MH Adult; Catecholamines/metabolism; Creutzfeldt-Jakob Syndrome/*etiology/*physiopathology; Female; Ganglia, Sympathetic/metabolism; Human; Magnetic Resonance Imaging; Neurons/*metabolism/pathology; PrPsc Proteins/*metabolism; Stellate Ganglion/metabolism; Support, Non-U.S. Gov't; Sympathetic Nervous System/*metabolism/pathology
AD Raymond Escourolle Neuropathology Laboratory, Association Claude Bernard and INSERM U360, Pitie-Salpetriere Hospital, 75013, Paris, France. haik@mailhost.chups.jussieu.fr
SP englisch
PO USA